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Childhood Gastrointestinal Stromal Tumor

MedGen UID:
906937
•Concept ID:
C4086159
•
Neoplastic Process
Synonym: Childhood GIST

Definition

A gastrointestinal stromal tumor (GIST) occurring in childhood. Pediatric GISTs differ biologically from adult GISTs in that only 11% of pediatric GISTs have activating mutations of KIT and PDGFRA. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood Gastrointestinal Stromal Tumor

Professional guidelines

PubMed

Current status of the treatment of gynecologic cancer by site: ovary.
Barber HR, Kwon TH
Cancer 1976 Jul;38(1 SUPPL):610-9. doi: 10.1002/1097-0142(197607)38:1<610::aid-cncr2820380186>3.0.co;2-t. PMID: 1277113

Recent clinical studies

Etiology

Gastroduodenal intussusception caused by gastric gastrointestinal stromal tumor in adults: a case report and literature review.
Zhang W, Chen H, Zhu L, Kong Z, Wang T, Li W
J Int Med Res 2022 May;50(5):3000605221100772. doi: 10.1177/03000605221100772. PMID: 35632985Free PMC Article
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Von Hippel-Lindau and Hereditary Pheochromocytoma/Paraganglioma Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.
Rednam SP, Erez A, Druker H, Janeway KA, Kamihara J, Kohlmann WK, Nathanson KL, States LJ, Tomlinson GE, Villani A, Voss SD, Schiffman JD, Wasserman JD
Clin Cancer Res 2017 Jun 15;23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547. PMID: 28620007
The role of complex II in disease.
Hoekstra AS, Bayley JP
Biochim Biophys Acta 2013 May;1827(5):543-51. Epub 2012 Nov 20 doi: 10.1016/j.bbabio.2012.11.005. PMID: 23174333
Gastrointestinal stromal tumor: a very rare cause of jejunoileal intussusception in a 6-year-old girl.
Gunaydin M, Biçakci U, Bozkurter AT, Elli M, Tander B, Bariş S, Rizalar R, Aritürk E, Bernay F
J Pediatr Surg 2012 May;47(5):E15-8. doi: 10.1016/j.jpedsurg.2011.12.021. PMID: 22595603

Diagnosis

Gastroduodenal intussusception caused by gastric gastrointestinal stromal tumor in adults: a case report and literature review.
Zhang W, Chen H, Zhu L, Kong Z, Wang T, Li W
J Int Med Res 2022 May;50(5):3000605221100772. doi: 10.1177/03000605221100772. PMID: 35632985Free PMC Article
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Von Hippel-Lindau and Hereditary Pheochromocytoma/Paraganglioma Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.
Rednam SP, Erez A, Druker H, Janeway KA, Kamihara J, Kohlmann WK, Nathanson KL, States LJ, Tomlinson GE, Villani A, Voss SD, Schiffman JD, Wasserman JD
Clin Cancer Res 2017 Jun 15;23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547. PMID: 28620007
Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113
Beyond the GIST: mesenchymal tumors of the stomach.
Kang HC, Menias CO, Gaballah AH, Shroff S, Taggart MW, Garg N, Elsayes KM
Radiographics 2013 Oct;33(6):1673-90. doi: 10.1148/rg.336135507. PMID: 24108557Free PMC Article

Therapy

Gastroduodenal intussusception caused by gastric gastrointestinal stromal tumor in adults: a case report and literature review.
Zhang W, Chen H, Zhu L, Kong Z, Wang T, Li W
J Int Med Res 2022 May;50(5):3000605221100772. doi: 10.1177/03000605221100772. PMID: 35632985Free PMC Article
Gastrointestinal stromal tumors (GIST) in children and adolescents: A comprehensive review of the current literature.
Benesch M, Wardelmann E, Ferrari A, Brennan B, Verschuur A
Pediatr Blood Cancer 2009 Dec 15;53(7):1171-9. doi: 10.1002/pbc.22123. PMID: 19499582
Current status of the treatment of gynecologic cancer by site: ovary.
Barber HR, Kwon TH
Cancer 1976 Jul;38(1 SUPPL):610-9. doi: 10.1002/1097-0142(197607)38:1<610::aid-cncr2820380186>3.0.co;2-t. PMID: 1277113

Prognosis

Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113
Succinate dehydrogenase (SDH) and mitochondrial driven neoplasia.
Gill AJ
Pathology 2012 Jun;44(4):285-92. doi: 10.1097/PAT.0b013e3283539932. PMID: 22544211
Gastrointestinal stromal tumors (GIST) in children and adolescents: A comprehensive review of the current literature.
Benesch M, Wardelmann E, Ferrari A, Brennan B, Verschuur A
Pediatr Blood Cancer 2009 Dec 15;53(7):1171-9. doi: 10.1002/pbc.22123. PMID: 19499582
C-kit expression and mutational analysis in medulloblastoma.
Chilton-Macneill S, Ho M, Hawkins C, Gassas A, Zielenska M, Baruchel S
Pediatr Dev Pathol 2004 Sep-Oct;7(5):493-8. Epub 2004 Jul 30 doi: 10.1007/s10024-004-1116-7. PMID: 15547773
Bone metastasizing renal tumor of childhood. A clinicopathological study of eleven cases from the Prague Pediatric Tumor Registry.
Kodet R, Stejskal J, Malis J, Horák J
Pathol Res Pract 1994 Sep;190(8):750-8. doi: 10.1016/S0344-0338(11)80421-8. PMID: 7831150

Clinical prediction guides

Neurofibromatosis type 1 (NF1) and associated tumors.
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113
Succinate dehydrogenase (SDH) and mitochondrial driven neoplasia.
Gill AJ
Pathology 2012 Jun;44(4):285-92. doi: 10.1097/PAT.0b013e3283539932. PMID: 22544211
Gastrointestinal stromal tumour of the duodenum in childhood: a rare case report.
Chiarugi M, Galatioto C, Lippolis P, Zocco G, Seccia M
BMC Cancer 2007 May 9;7:79. doi: 10.1186/1471-2407-7-79. PMID: 17490483Free PMC Article
C-kit expression and mutational analysis in medulloblastoma.
Chilton-Macneill S, Ho M, Hawkins C, Gassas A, Zielenska M, Baruchel S
Pediatr Dev Pathol 2004 Sep-Oct;7(5):493-8. Epub 2004 Jul 30 doi: 10.1007/s10024-004-1116-7. PMID: 15547773
Bone metastasizing renal tumor of childhood. A clinicopathological study of eleven cases from the Prague Pediatric Tumor Registry.
Kodet R, Stejskal J, Malis J, Horák J
Pathol Res Pract 1994 Sep;190(8):750-8. doi: 10.1016/S0344-0338(11)80421-8. PMID: 7831150

Recent systematic reviews

Gastrointestinal stromal tumors (GIST) in children and adolescents: A comprehensive review of the current literature.
Benesch M, Wardelmann E, Ferrari A, Brennan B, Verschuur A
Pediatr Blood Cancer 2009 Dec 15;53(7):1171-9. doi: 10.1002/pbc.22123. PMID: 19499582

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